PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “LEIOMYOSARCOMA”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 55 records · Page 3Linked to original sources

Intermediate and fine filaments of vascular leiomyomas (angiomyoma), leiomyoma and leiomyosarcomas of large veins.

The expression of fine and intermediate filaments in 10 cases of leiomyosarcoma originating from a large vein, 9 cases of vascular leiomyoma (angiomyoma) and one case of a leiomyoma originating from the wall of the saphena magna vein was studied immunohistochemically by using 6 different anti-desmin antibodies, one anti-vimentin antibody and 2 antibodies to muscle-specific isoforms of actin. All the benign tumors and all the leiomyosarcomas of a large vein as well as the vein of origin were positively stained for desmin. The staining results obtained using the different anti-desmin antibodies varied considerably, however, and formaldehyde-fixed tissues were apparently inappropriate for some of them. No single anti-desmin antibody produced a positivity in all cases, and the extent and distribution of the positivity varied by being irregular and patchy in the leiomyosarcomas and in the muscle walls of the veins, while the benign tumors generally revealed a more uniform and strong positivity. Antibodies to muscle-specific and smooth muscle-specific actin produced a positive staining in all the benign tumors, as well as all the leiomyosarcomas and the veins from which they originated. A strong and uniform positivity was observed in the benign tumors and muscle walls of the veins, while the positivity in the leiomyosarcomas was more irregular, as it was for desmin. Vimentin was constantly expressed in the benign tumors and in the veins of origin, but only in 5/10 of the leiomyosarcomas. It is concluded from this study that the immunohistochemical demonstration of desmin, utilizing a monoclonal antibody appropriate to the type of fixation used, and muscle specific isoforms of actin, provide strong support to the light-microscopic diagnosis of leiomyosarcoma of venous origin.

Actins↗

Array comparative genomic hybridization reveals distinct DNA copy number differences between gastrointestinal stromal tumors and leiomyosarcomas.

Leiomyosarcomas are spindle cell tumors showing smooth muscle differentiation. Until recently, most gastrointestinal stromal tumors (GIST) were also classified as smooth muscle tumors, but now GISTs are recognized as a separate entity, defined as spindle cell and/or epithelioid tumors localized in the gastrointestinal tract. Using microarray-based comparative genomic hybridization (array CGH), we have created a detailed map of DNA copy number changes for 7 GISTs and 12 leiomyosarcomas. Considerable gains and losses of chromosomal segments were observed in both tumor types. The most frequent aberration observed in GISTs was loss of chromosomes 14 and 22, with minimal recurrent regions in 14q11.2-q32.33 (71% of the tumors) and 22q12.2-q13.31 (100%). In leiomyosarcomas, frequent loss of chromosome 10 and 13q was observed, with minimal recurrent regions in 10q21.3 (75%) and 13q14.2-q14.3 (75%). Recurrent high-level amplification of 17p13.1-p11.2 was detected in leiomyosarcomas. Expression profiling using cDNA microarrays revealed four candidate genes in this region with high expression (AURKB, SREBF1, MFAP4, and FLJ10847). Altered expression of AURKB and SREBF1 has been observed previously in other malignancies. Hierarchical clustering of all samples separated GISTs and leiomyosarcomas into two distinct clusters. Statistical analysis identified six chromosomal regions, 1p36.11-p13.1, 9q21.11-9q34.3, 14q11.2-q23.2, 14q31.3-q32.33, 15q24.3-q26.3, and 22q11.21-q13.31, which were significantly different in copy number between GISTs and leiomyosarcomas. Our results show the potential of using array comparative genomic hybridization to classify histologically similar tumors such as GISTs and leiomyosarcomas.

Adult↗

Detection of chromosomal imbalances in leiomyosarcoma by comparative genomic hybridization and interphase cytogenetics.

Leiomyosarcomas comprise a group of malignant soft-tissue tumors with smooth-muscle differentiation. In this study, 14 cases of leiomyosarcoma were screened for changes in relative chromosome copy number by comparative genomic hybridization. A high number of imbalances (mean, 16.3; range, 6-26) was detected, with chromosomal gains occurring about twice as much as losses. The most frequent gains were found in 5p15, 8q24, 15q25-->q26, 17p, and Xp (43% to 50%), whereas the most frequent losses were found in 10q and 13q (50% and 78%, respectively). Twenty high-level amplifications affecting 15 different chromosomal subregions were detected in nine different tumors. In three leiomyosarcomas, sequences on chromosome arm 17p were found to be highly amplified, with a minimal overlapping region on subbands 17p12-->p11. We further discovered that the Smith-Magenis syndrome critical region on 17p11.2 is included in the 17p amplicons of two leiomyosarcoma cases. Using probes flanking this genetically unstable region, a mean of 14 and 22 signals per nucleus, respectively, was detected in both leiomyosarcomas by fluorescence in situ hybridization. In conclusion, this analysis identifies a number of characteristic chromosomal imbalances in leiomyosarcomas and provides evidence for the localization of potential oncogenes and tumor suppressor genes active in leiomyosarcoma genomes.

Adult↗

Superficial leiomyosarcoma of the head and neck: case report and review of the literature.

Superficial leiomyosarcomas are rare in the head and neck region. Because of the infrequent nature of soft tissue sarcomas in general, superficial leiomyosarcomas are often misdiagnosed on clinical grounds. Immunohistochemistry is essential for an accurate histologic diagnosis, and it should include a broad panel of antibody studies. With respect to differences in clinical appearance and biologic behavior, superficial leiomyosarcomas can be broadly classified as either cutaneous or subcutaneous; local control and overall survival are significantly more favorable in patients with the former. The primary treatment of a leiomyosarcoma is a wide surgical excision with an emphasis on negative margins. Treatment failures are usually attributable to a local recurrence. Systemic metastasis occurs in about one-third of patients with subcutaneous involvement. Although cutaneous leiomyosarcoma is considered a relatively more benign process with minimal metastatic potential, systemic metastasis is still possible. This was demonstrated in our case, as a recurrent cutaneous leiomyosarcoma metastasized to the lung. Proper management requires inclusion of this entity in the differential diagnosis, as well as familiarity with its clinical behavior. In this article, we review the literature on superficial leiomyosarcoma and discuss its epidemiology, presentation, clinical behavior, evaluation, tissue diagnosis, staging, and treatment.

Cyclosporine↗

[Metastatic lung tumor from uterine leiomyosarcoma].

We herein present 2 cases of metastatic lung tumor derived from uterine leiomyosarcoma. In the case 1, a 59-year-old woman was admitted to our hospital to examine abnormal shadow detected on chest X-ray. She had undergone hysterectomy and oophorectomy for uterine leiomyosarcoma 19 months previously. A round 3 cm mass in the right lung (S10) was seen on chest X-ray and computed tomography (CT). No other distant metastases or local recurrence were found, and the right lower lobectomy was perfomed under the clinical diagnosis of metastatic lung tumor. Postoperative pathologic examination revealed the tumor as a metastatic leiomyosarcoma. The patient recovered uneventfully, and there have been no signs of recurrence for 26 months after the pulmonary resection. In the case 2, a 58-year-old woman, who had undergone hysterectomy and oophorectomy for uterine leiomyosarcoma 7 months previously, was admitted to our hospital for further examination of pulmonary tumors on chest X-ray. Two tumors were recognized in the left lung (S8 and S10) on chest X-ray and CT. No other distant metastases or local recurrence were found, and the left lower lobectomy was performed under the clinical diagnosis of metastatic lung tumors. Pathological examinations revealed smooth muscle cells with nuclear pleomorphism and high mitotic indices. The tumors proved to be lung metastases derived from uterine leiomyosarcoma. Postoperative course was uneventful. However, brain metastasis was found 1 month after the pulmonary resection, and she underwent resection of brain metastasis. Two months after the brain metastasectomy, local recurrence of the brain tumor developed and re-resection followed by stereotactic radiotherapy was performed. Furthermore, intrapelvic recurrence was found 4 months after the pulmonary resection. Exploratory laparotomy revealed the tumor was unresectable, and she received 4 courses of chemotherapy (paclitaxel and carboplatin). For metastatic lung tumor from uterine leiomyosarcoma, surgery has been considered the best choice. However, for patients with uterine leiomyosarcoma who cannot be treated surgically because of multiple metastatic tumors or poor surgical risk chemotherapy (paclitaxel and carboplatin) or stereotactic radiotherapy can be strategies.

Antineoplastic Combined Chemotherapy Protocols↗

Tumor specific Epstein-Barr virus infection is not associated with leiomyosarcoma in human immunodeficiency virus negative individuals.

BACKGROUND: Recent studies have suggested that the Epstein-Barr virus (EBV) is associated with leiomyosarcoma in children with human immunodeficiency virus (HIV) and in organ transplant recipients. To determine whether EBV is associated with leiomyosarcoma in HIV negative patients, the authors examined resected leiomyosarcomas for EBV and HIV. METHODS: Twenty-four leiomyosarcomas were studied and their diagnosis confirmed on pathologic review. From these specimens DNA was isolated. Tumor samples were analyzed for EBV and HIV using a polymerase chain reaction (PCR) technique followed by gel electrophoresis and Southern blot analysis. DNA from an EBV-infected human Burkitt's lymphoma cell line and peripheral blood from an HIV positive patient were used as positive controls for the presence of EBV and HIV, respectively. Immunohistochemistry was performed using an antibody to Epstein-Barr nuclear antigen. RESULTS: HIV was not present in any of the patients analyzed. EBV DNA was detected in tumor tissue; however, 80 cycles of PCR were used before EBV sequences were detected. Therefore, the data indicate that tumor tissue was not infected with EBV. The positive results observed after 80 cycles of PCR were likely due to infiltrating lymphocytes. Immunohistochemistry confirmed the lack of active or latent EBV infection in tumor cells. CONCLUSIONS: The results indicate that EBV is not associated with sporadic leiomyosarcoma in HIV negative patients. Therefore, the biology of leiomyosarcoma associated with HIV may be substantially different from the more common sporadic form.

Adult↗

Development of leiomyosarcoma of the uterus in MMTV-CR-1 transgenic mice.

Overexpression of Cripto-1 (CR-1) in FVB/N mice using the MMTV-LTR promoter results in increased mammary tumourigenesis in these female transgenic mice (MMTV-CR-1). Here, we characterize uterine tumours that developed in 15/76 (19.7%) of MMTV-CR-1 female nulliparous or multiparous mice during 24 months of observation compared with 0/33 (0%) of FVB/N normal control mice observed during the same time period (p < 0.01). The uterine tumours collected from the MMTV-CR-1 mice were classified as leiomyosarcomas and found to express the CR-1 transgene by polymerase chain reaction analysis and immunohistochemistry. Detection by western blot analysis showed higher levels of phosphorylated (P) forms of c-src, Akt, GSK-3beta, and dephosphorylated (DP)-beta-catenin in lysates from MMTV-CR-1 uterine leiomyosarcomas in comparison with lysates from normal control FVB/N uteri. Immunostaining showed increased nuclear localization of beta-catenin in the MMTV-CR-1 uterine leiomyosarcomas. Increased immunostaining for CR-1 was detected in 9/13 (69.2%) cases of human leiomyosarcoma compared with staining in 3/15 (20%) human leiomyoma sections. Stronger immunostaining for P-src, P-Akt, P-GSK-3beta and increased nuclear localization of beta-catenin was also seen in human leiomyosarcomas in comparison with leiomyomas. Normal human uterine smooth muscle (UtSM) cells treated with exogenous soluble rhCR-1 showed increased levels of P-src, P-Akt, P-GSK-3beta and dephosphorylated (DP)-beta-catenin and increased proliferation (p < 0.05) and migration (p < 0.01) in comparison with untreated control UtSM cells. Inhibitors against c-src, Akt or beta-catenin, individually or in combination, significantly reduced CR-1-induced migration. These results suggest a role for CR-1 during uterine tumourigenesis either directly by activating c-src and Akt and/or via cross-talk with the canonical Wnt signalling pathway, as suggested by the increased expression of P-GSK-3beta, DP-beta-catenin, and increased nuclear localization of beta-catenin in human and MMTV-CR-1 mice leiomyosarcomas.

Animals↗

Bcl-2 expression in a primary leiomyosarcoma of the ovary: a case report.

BACKGROUND: Primary ovarian leiomyosarcoma is an extremely rare malignant smooth-muscle neoplasm with fewer than 30 documented cases worldwide. Immunohistochemically, ovarian leiomyosarcomas are characterized by the expression of alpha-smooth muscle actin (alpha-SMA). Until recently, no report investigated the role of bcl-2 expression in primary ovarian leiomyosarcoma. We report the management and an immunohistochemical analysis of bcl-2 in a patient with primary leiomyosarcoma of the ovary. CASE REPORT: The patient, a 71-year-old woman, presented with a history of lower abdominal pain and a palpable mass in the lower abdomen. Surgical exploration revealed a large left adnexal mass that had developed from the left ovary and infiltrated one part of the omentum adherent to the left adnexa. Microscopically the left adnexal tumor consisted of spindle cells arranged in smooth-muscle bundles and fascicles. The tumor was characterized by high cellularity and nuclear polymorphism, as well as patchy necrosis and large areas of hemorrhage. Mitotic activity exceeded 5 mitoses per 10 high-power fields with many atypical mitoses. The tumor appeared encapsulated, and the capsule was partially disrupted by tumor cells. Immunohistochemically, the tumor showed a strong staining reaction for muscle actin, alpha-SMA and neuron-specific enolase, and also a weak reaction for vimentin. Strong staining of bcl-2 was detected, with more than 80% of sarcoma cells positive. According to the FIGO staging system for ovarian cancer, the tumor was classified as a primary ovarian leiomyosarcoma stage III C, and the patient was treated with an adjuvant chemotherapy regimen consisting of cisplatin and ifosfamide. Two days after the third cycle of cisplatin/ifosfamid she developed an apoplexy spontaneously and chemotherapy was therefore discontinued. The patient remained without evidence of disease until she died as a result of intercurrent pneumonia 14 months after initial surgery. CONCLUSIONS: Beside the routine histological and immunohistochemical characteristics of primary ovarian leiomyosarcoma, strong staining for bcl-2 was detected. In order to understand more about the nature and the behaviour of this highly malignant neoplasm and to be able to improve the treatment, the prognostic value of bcl-2 has to be investigated in additional studies.

Aged↗

Surgical management of gastric leiomyosarcoma: evaluation of the propriety of laparoscopic wedge resection.

Laparoscopic surgery has been applied to malignant gastric tumors. To evaluate the propriety of laparoscopic wedge resection for gastric leiomyosarcoma it is necessary to question whether lymph node dissection is necessary for the surgical management of gastric leiomyosarcoma. A retrospective study on open surgery cases of gastric leiomyosarcoma was performed to address this issue. The clinical records of 28 patients with gastric leiomyosarcoma who had had surgery were examined. The patients who underwent open surgery were divided into a systematic lymph node dissection (SLND) group (n = 9) and a nondissection (non-D) group (n = 19). No patient had lymph node metastasis at the time of operation or recurrence, and statistical analysis showed no difference between the SLND and non-D groups in terms of survival rates. These data suggest that SLND might not be necessary for the surgical management of gastric leiomyosarcoma and that laparoscopic wedge resection of the stomach can be considered a first-line treatment for gastric leiomyosarcoma.

Adult↗

[Misdiagnosis of a Leiomyosarcoma of the inferior vena cava as a renal cell carcinoma].

Leiomyosarcoma of the vena cava inferior (VCI) is a rare mesenchymal malignant tumor of the retroperitoneum. About 200 cases have been described in the literature so far. Leiomyosarcomas may resemble other tumors including renal cell carcinoma, adrenal carcinoma, and hepatic adenoma. Thus, misinterpretation of a leiomyosarcoma of the VCI is a common problem. We present a 71-year-old female with a large subhepatic, retroperitoneal tumorous mass which was diagnosed to be a renal cell carcinoma by sonography and computed tomography. After application of magnetic resonance imaging and retrospective analysis of the CT scan, diagnosis could be revised. Laparotomy and en bloc resection of the tumor was performed; histopathological examination confirmed a leiomyosarcoma of the VCI. The postoperative course was unremarkable. Leiomyosarcoma of the VCI may resemble advanced stages of renal cell carcinoma. High-resolution imaging modalities (computed tomography, magnetic resonance imaging) are able to precisely evaluate the typical imaging characteristics of leiomyosarcomas, thus determining correct diagnosis in affected patients. This is essential for successful operative therapy.

Aged↗

Leiomyosarcomas of the oral tissues: clinicopathologic analysis of 50 cases.

PURPOSE: Primary oral leiomyosarcomas are rare tumors. Information regarding the biological behavior, prognosis, and appropriate management of this neoplasm is lacking in the literature. The purpose of this report was to summarize the data of isolated case reports of primary oral leiomyosarcoma that have been published in the English literature during the past 25 years. The cases of 4 additional new patients who have been treated in our department during the past 10 years are also presented. PATIENTS AND METHODS: The data for 46 patients obtained from 32 individual articles retrieved from the English literature were added to our 4 cases and produced a total number of 50 cases of primary leiomyosarcomas of the oral tissues. Patients were analyzed according to demographic data, anatomic location, type of treatment, and survival. RESULTS: Primary oral leiomyosarcoma may affect any age with peaks of occurrence in the third, sixth, and seventh decades of life. There is no gender predilection. Female patients presented the higher incidence in the third decade, whereas males had an even age distribution. The tumor arises in approximately 70% of the cases in the maxillary and mandibular bones. Radical surgery was the treatment of choice. Radiotherapy and chemotherapy when applied in recurrent tumors had little effect. The most adverse prognostic factor was the positive surgical margins. The 5-year survival was 62% (62.9% for females and 52.6% for males, P > .1968). CONCLUSIONS: Cases of oral leiomyosarcoma appear to be associated with major neurovascular structures of the facial skeleton, as evidenced from the imaging studies of our 4 patients. Primary oral leiomyosarcoma is a rare tumor that should be managed with aggressive surgical resection in order to safeguard curability. Histopathologic diagnosis is greatly facilitated with positive immunohistochemical staining for smooth muscle antigenic markers.

Adolescent↗

Principles in the management of pediatric intestinal leiomyosarcomas.

Primary intestinal leiomyosarcomas in children are unusual tumors with little known about their natural history. Two patients (aged 9 and 10 years) with leiomyosarcoma of the jejunum treated at our institution are reported and added to the 20 other patients reported in the literature. The first patient had a grade 1 leiomyosarcoma completely resected, received no additional therapy, and remains alive with no evidence of disease 14 years later. The second patient presented with a perforated grade 2 leiomyosarcoma which was incompletely resected; he received combination chemotherapy with an initial good response, but eventually died from sarcomatosis 7 years after initial diagnosis. The 22 cases of pediatric intestinal leiomyosarcoma presented more commonly with obstruction or perforation, were able to be completely resected more often, and appear to have a better prognosis than in adults. Thus, intestinal leiomyosarcomas in children appear to have a natural history different from that of the same tumor arising in adults.

Child↗

Paratesticular rhabdomyosarcomas and leiomyosarcomas: a clinicopathological review.

One case of embryonal paratesticular sarcoma and 2 cases of leiomyosarcomas are reported. The 13-year-old boy with embryonal sarcoma is well after an orchiectomy and high ligation of the spermatic cord followed by radiotherapy and chemotherapy. A 66-year-old man has been doing well after orchiectomy and hemiscrotectomy for a paratesticular leiomyosarcoma. The third patient had a highly pleomorphic leiomyosarcoma and died 2 months postoperatively. The clinical and histological diagnosis of paratesticular rhabdomyosarcomas and leiomyosarcomas is reviewed and the various therapeutic approaches to these neoplasms are discussed. Retroperitoneal lymph node dissection, radiotherapy and chemotherapy are important adjuncts to orchiectomy in the management of rhabdomyosarcomas. On the contrary, retroperitoneal lymph node dissection and radiotherapy are not indicated in leiomyosarcomas, since these neoplasms tend to metastasize by the hematogenous route and are radioresistant. At the present time we are unable to evaluate chemotherapy in the management of paratesticular leiomyosarcomas.

Aged↗

Primary gingival leiomyosarcoma. A clinicopathological study of 1 case with prolonged survival.

BACKGROUND: Leiomyosarcoma is a relatively uncommon mesenchymal tumor that exhibits smooth-muscle differentiation. Only 3 to 10% of leiomyosarcomas arise in the head and neck, the nose and paranasal sinuses, skin and subcutaneous tissue and cervical esophagus being the most common localizations. Most leiomyosarcomas involving the oral tissues primarily affect the maxillary sinus, the maxillary or mandibular bone. A review of the English-language literature since 1908 revealed 30 reported cases of primary leiomyosarcoma of the oral mucosa and soft tissues. MATERIAL AND METHODS: We report on a case of gingival leiomyosarcoma, arising in a 31-year-old female and involving the upper alveolar mucosa. Following the diagnosis of malignant neoplasm on frozen sections and an en-block resection, the tumour was formalin-fixed and paraffin embedded for histological and immunohistochemical examination. RESULTS: Microscopically, the tumor was composed of interlacing fascicles of spindle-shaped cells with elongated, blunt-ended nuclei and eosinophilic cytoplasm, containing PAS-positive granules. Mitoses, both typical and atypical, and scattered necrotic foci were present. Consistent desmin, muscle specific and alpha-smooth muscle-specific, and vimentin immunoreactivity was demonstrated in the tumor cells. The patient is alive and free of disease at a 7-year follow-up. CONCLUSIONS: Intra-oral leiomyosarcomas are exceptionally rare. Accurate diagnosis and treatment is largely based on the careful search of clinical signs indicative of malignancy (e.g., neoplastic bone destruction, wide invasion of adjacent tissues) and intra-operative (frozen sections) examination of the lesion. Though the case reported herein showed an attenuated clinical behavior, prolonged follow-up is mandatory in view of possible tumor relapse.

Actins↗

Possible role of calponin h1 as a tumor suppressor in human uterine leiomyosarcoma.

BACKGROUND: Calponin h1, a basic actin-binding protein capable of inhibiting smooth muscle contraction, is a constitutive element of smooth muscle cells. However, in leiomyosarcoma (a type of smooth muscle neoplasm of the uterus), reduced expression of calponin h1 is observed, as we have reported previously. In this study, we sought to assess the effects (in vitro and in vivo) of increasing calponin h1 expression in leiomyosarcoma cells. METHODS: A plasmid containing a human calponin h1 complementary DNA and a bacterial neomycin-resistance gene was transfected into the human leiomyosarcoma cell lines SKN and SK-LMS-1 by electroporation. Southern blotting, reverse transcription-polymerase chain reaction analysis, western blotting, and immunohistochemistry were used to confirm DNA transfer and expression of the calponin h1 protein in neomycin-resistant clones. We characterized the morphology of calponin h1-transfected cells, and we evaluated their proliferative activity and tumorigenicity by use of a 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl-2H-tetrazolium bromide assay, an anchorage-independent growth assay, and a nude mouse tumorigenicity assay. RESULTS: The morphology of calponin h1-transfected cells in culture resembled that of cultured normal myometrial smooth muscle cells. With SK-LMS-1 cells, proliferation of calponin h1-transfection cells was reduced to 69% of control; with SKN cells, calponin h1 transfection reduced proliferation to 70% of control. In assays of anchorage-independent growth and in vivo tumorigenicity, both growth and tumorigenicity were statistically significantly reduced in calponin h1-transfected leiomyosarcoma cells. CONCLUSIONS: Calponin h1 may function as a tumor suppressor in leiomyosarcoma. Clinically, transfer of a calponin h1 complementary DNA into poorly differentiated leiomyosarcoma cells may be of potential therapeutic value through induction of a normal, differentiated cellular phenotype.

Animals↗

Cutaneous leiomyosarcoma.

We report the clinical, histopathologic, immunohistologic, and prognostic findings in 19 patients with cutaneous leiomyosarcoma, eight males and 11 females (mean age, 66 years; age range, 41-93 years). The tumors presented mainly as solitary lesions and were located on the head and neck (eight lesions), trunk (four lesions), upper extremities (three lesions), and lower extremities (four lesions). Histopathologically, two predominant growth patterns were observed: nodular (12 cases) and diffuse (seven cases). Neoplasms with a nodular growth pattern were characterized by high cellularity and prominent nuclear atypia, and they showed conspicuous mitoses, several necrotic cells, and sometimes extensive necrotic areas. By contrast, most cutaneous leiomyosarcomas with a diffuse growth pattern revealed low cellularity, well-differentiated smooth muscle cells, inconspicuous mitotic figures, and few or no necrotic cells. Immunohistologic investigations revealed all cutaneous leiomyosarcomas to express vimentin and smooth muscle actin. Pan-muscle actin (HHF-35) was also expressed in most cases (15 lesions). However, only 12 lesions showed positive staining for desmin. Remarkable was the expression of cytokeratins in five lesions. Clinical follow-up revealed local recurrences in five patients (three cases with nodular pattern and two lesions with a diffuse pattern) after a period ranging from 8 months to 3 years after surgical excision. No distant metastases have been observed in our series. We conclude that cutaneous leiomyosarcoma with a diffuse growth pattern may constitute a pitfall in histopathologic diagnosis because of the presence of only subtle criteria for malignancy. Cutaneous leiomyosarcoma may show different immunophenotypes, thus emphasizing the importance of using a large panel of antibodies (smooth muscle actin, HHF-35, desmin, vimentin, cytokeratins, and S-100 protein) in immunohistologic diagnosis. Cutaneous leiomyosarcoma sometimes reveals local recurrences, but it has negligible potential for distant metastases.

Actins↗

Smooth muscle neoplasms of the urinary bladder: a clinicopathologic comparison of leiomyoma and leiomyosarcoma.

We report the clinicopathologic, immunohistochemical, and DNA ploidy findings of 18 leiomyosarcomas of the urinary bladder. In addition, we compare these malignant smooth muscle tumors with 10 cases of urinary bladder leiomyoma. The 14 male and four female patients with leiomyosarcoma ranged in age from 25 to 88 years (mean 64 years). The tumors ranged from 3.0 to 15.0 cm (mean 7.1 cm) in greatest dimension and were moderately to highly cellular, consisting of interlacing fascicles of spindled cells with mild to marked nuclear atypia. Coagulative tumor necrosis was identified in 14 cases (78%), and mitotic activity ranged from 1 to 42 mitotic figures (MF) per 10 high power fields (HPF) (mean 12 MF/10 HPF). Tumors were classified as either high-grade (12 cases) or low-grade (six cases) based on nuclear atypia, mitotic activity, and tumor necrosis. Actin positivity was present in 15 tumors (83%), and desmin immunoreactivity was present in seven tumors (39%). All cases were negative for epithelial markers and S-100. Proliferative activity, as assessed by MIB-1 staining, ranged from 0.1% to 51.4% (median 9.1%). Seven (39%) of the leiomyosarcomas were DNA aneuploid, eight (44%) were tetraploid, and three (17%) were diploid. Five patients underwent radical cystoprostatectomy, one radical cystectomy, seven had partial cystectomy, two underwent pelvic exenteration, and three patients had transurethral resection only. Follow-up information was available on all 18 cases and ranged from 2 to 68 months (mean 22 months). Of the 12 patients with high-grade tumors, six (50%) died of disease from 2 to 20 months (mean 7 months) after diagnosis and three patients (25%) are alive with metastatic tumor. Two of the six patients with low-grade leiomyosarcoma died of tumor, 61 and 68 months after diagnosis. There were five male and five female patients with leiomyoma ranging in age from 22 to 78 years (mean 61 years). The tumors ranged from 0.5 to 4.5 cm (mean 1.6 cm) in greatest dimension, were well circumscribed, and had low cellularity. Mitotic activity, necrosis, and cellular atypia were absent, and the tumors were strongly positive for both actin and desmin. MIB-1 staining ranged from 0% to 3.8% (median 0.8%). Seven (87.5%) of the leiomyomas were DNA diploid or near-diploid and one (12.5%) was DNA aneuploid. Six patients were treated with transurethral resection and four with partial cystectomy. All 10 patients were alive at the last follow-up (mean follow-up 75 months), and no tumor recurred or metastasized. Our study shows that low-grade leiomyosarcomas are capable of malignant behavior, and high-grade leiomyosarcomas appear to behave more aggressively than low-grade tumors. In addition, the diagnosis of urinary bladder leiomyoma should be reserved for noninfiltrative smooth muscle tumors lacking mitotic activity, cytologic atypia, and necrosis.

Actins↗

Leiomyosarcoma of the orbit. A case report and review of the literature.

Leiomyosarcoma has been rarely encountered in the orbit. Leiomyosarcoma may arise as a primary tumor in the orbit, metastasize from distant sites to the orbit, extend into the orbit from the paranasal sinuses, or appear after radiation therapy to the orbit. The 43-year-old female patient described in this paper underwent excision of a leiomyosarcoma in the subcutaneous tissue of the buttock. Seven years later, a leiomyosarcoma in the medial orbit was removed. The clinical course of this patient's disease was similar to that of other subcutaneous leiomyosarcomas documented in the literature. Gynecological history was remarkable for a leiomyoma of the uterus, an interesting association found in at least one other case in the literature. This was the third documented case of metastatic leiomyosarcoma in the orbit.

Adult↗